Daniel J. Santa Cruz

Connective tissue nevi of the skin: Clinical, genetic, and histopathologic classification of hamartomas of the collagen, elastin, and proteoglycan type

Connective tissue nevi of the skin are hamartomatous lesions consisting predominantly of one of the components of the extracellular matrix, namely, collagen, elastin, or glycosaminoglycans. On the basis of clinical, histopathologic, and genetic considerations, the connective tissue nevi can be classified into defined categories. Association with extracutaneous features allows further delineation of these disease entities and aids in establishing an accurate diagnosis.

Aneurysmal (“Angiomatoid”) fibrous histiocytoma of the skin

Seventeen cases are reported of a variety of cutaneous fibrous histiocytoma, which we have designated as aneurysmal (“angiomatoid”) fibrous histiocytoma. These lesions differ from the classical cutaneous fibrous histiocytoma in both their clinical presentation and pathologic features.

Targetoid hemosiderotic hemangioma

We describe a vascular lesion with characteristic clinical and histologic features. The patients when first seen have a small, single, annular, targetoid-appearing lesion. Histologically it is a noncircumscribed vascular proliferation that may extend into the subcutaneous tissue. The earliest finding appears to be a superficial proliferation of ectatic dermal vascular lumina with intraluminal papillary projections. The endothelial cells are flat or conspicuously epithelioid with solid intraluminal projections. The deeper component is composed of angular, lymphatic-like lumina that concentrate around sweat gland coils, often making small hemangiomatous nodules. Extensive red cell extravasation, inflammatory aggregates, and fibrin thrombi are present. In later stages there is extensive stromal hemosiderin deposition. The endothelial cells are weakly positive for factor VIII-related antigen and strongly positive for Ulex europaeus 1 lectin. The lesion appears to be persistent but self-limited. While appearing clinically benign, it exhibits worrisome histologic features. The nosologic designation of this lesion is uncertain, but it shares certain morphologic features with epithelioid (histiocytoid) hemangioma and progressive lymphangioma. It also poses serious differential diagnostic problems with the early phases of Kaposis sarcoma.

Morphea and lichen sclerosus et atrophicus: Clinical and histopathologic studies in patients with combined features

Ten patients with skin lesions clinically consistent with morphea and lichen sclerosus et atrophicus were studied. In all cases, histopathologic changes of both morphea and lichen sclerosus et atrophicus were also present. The coexistence of morphea and lichen sclerosus et atrophicus in the same patient suggests that these lesions represent a spectrum which may reflect similar etiologic events or closely related pathologic processes in these two diseases.

The histologic spectrumof the cutaneous mycobacterioses

The authors examined the histopathology of cutaneous involvement in 31 cases of nonleprous mycobacterial infection. Cases include three patients with Mycobacterium kansasii infection, two with M. fortuitum infections, and one each with M. marinum and M. chelonei infections, as well as 18 with M. tuberculosis infections. In the remainder, species were not identified. The histopathologic picture was variable and often did not suggest mycobacterosis. The authors identified seven basic pathologic patterns of skin involvement: 1) abscess, 2) well-formed (tuberculoid) granulomas, 3) diffuse histiocytic infiltration, 4) panniculitis, 5) nonspecific chronic inflammation, 6) naked (sarcoidal) granulomas, and 7) rheumatoid-like nodules. Intermediate forms were also found. Some cases showed adnexal or epidermal involvement, while others showed variably distributed dermal infiltration. The results indicate that a wide variety of cutaneous, clinical, and histologic guises may be assumed by mycobacterial infections in normal and immunocompromised hosts.

Kaposi's sarcoma: Relationship with hematologic, lymphoid, and thymic neoplasia

Four cases of Kaposis sarcoma (KS) are described; one each was associated with Hodgkins disease, a thymoma, an atypical lymphoid proliferation resembling Castlemans disease, and a cutaneous reticulosis. The association of KS with lymphoproliferative, hematologic, and other neoplasms is thoroughly reviewed, with special attention to tissue diagnosis, relative order of development, and relationship to immunosuppressive treatment. Review of 65 accepted cases, including ours, showed that 85% of patients with KS and a known order of development appeared with KS either simultaneously or after the second neoplasm. There was a disproportionate tendency for hematologic malignancy to develop simultaneously with KS. Of the 22 patients who had KS subsequent to a second neoplasm, 18 (82%) had received some form of interval immunosuppression. This evidence and observations in renal allograft recipients, patients with pemphigus, and patients with other autoimmune disorders point to the frequency of a common background of deranged immune status in patients with KS. Therefore, either naturally occurring or iatrogenic immune deficiency likely predisposes patients to KS. Some possible mechanisms for this phenomenon are reviewed. Cancer 47:963–973, 1981.

Verrucous carcinoma. A light and electron microscopic, autoradiographic, and immunofluorescence study

Verrucous carcinomas of the rectum, plantar surface of the foot, and oral cavity were studied by means of light and electron microscopy, and autoradiographic and immunofluorescent techniques. Histologic examination showed that each tumor was composed mainly of mature squamous epithelium, and each had foci of slight cellular atypia. The cells in S‐phase consistently were situated near the basal layer. Immunofluorescent examination with antibasement membrane antibody showed areas of marked focal thickening and other areas where basement membrane was absent. Ultrastructural examination showed reduplicated as well as normal basal lamina. Numerous interdigitating microvilli and well developed desmosomes characterized the cells above the basal layer. A proliferative basal zone underlying a thick layer of well differentiated nonproliferating keratinocytes and reduplicated basal lamina were seen in all tumors, regardless of location. These consistent findings constitute evidence that verrucous carcinoma is a morphologic and cytokinetic entity that may occur in multiple anatomic sites.

Verruciform xanthoma. Occurrence in eroded skin in a patient with recessive dystrophic epidermolysis bullosa

Verruciform xanthoma is a rare, granular, plaque-like lesion which occurs on mucosal surfaces and is characterized by acanthosis with xanthoma cells in the dermis between rete pegs. A case of verruciform xanthoma is reported which occurred on the sacral region in a patient with recessive dystrophic epidermolysis bullosa (RDEB). Electron microscopic studies suggested that many of the xanthoma cells were fibroblasts whose vacuolated appearance was expressed in cell culture and persisted for several passages. The occurrence of a verruciform xanthoma in a patient with severe recessive dystrophic epidermolysis bullosa suggests that repeated epidermal and/or dermal damage gave rise to a reactive process involving the formation of lipid-laden cells.

Angiomatosis with luminal cryoprotein deposition

BACKGROUND Angioendotheliomatosis has recently been separated into two types. Malignant angioendotheliomatosis is a lymphoma in which the neoplastic cells accumulate in vascular lumens. Reactive angioendotheliomatosis is a proliferation of capillaries that is less well characterized. OBJECTIVE Our purpose was to describe the clinical and histopathologic features of three cases of reactive angiomatosis in patients with cryoproteinemia. METHODS Clinical and histologic data were correlated. Immunoperoxidase staining and electron microscopy were used in the histologic evaluation. RESULTS All three cases showed tufts of capillaries with luminal deposits of cryoproteins. Immunohistochemical studies showed that the cellularity of the tufts was primarily caused by a proliferation of pericytes. CONCLUSION Cryoproteinemia can cause angiomatosis that simulates a vascular neoplasm.

Febrile Ulceronecrotic Mucha‐Habermann's Disease with Interstitial Pneumonitis

A case of febrile ulceronecrotic Mucha‐Habermanns is presented. This disorder is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by the sudden eruption of diffuse coalescent ulcerations associated with high fever. In the present case the disease was preceded by the milder typical form of PLEVA. Histologically a leukocytoclastic vasculitis was seen in addition to the usual lymphocytic perivascular and lichenoid infiltrate. During the course of the disease the patient developed an interstitial pneumonitis which resolved concomitantly with the cutaneous lesions. Adenovirus type II recovered at the height of the illness from the patients urine may have etiologic implications in the pathogenesis of the disease.